ECHD1

Ethylmalonyl-CoA decarboxylase; Isoform 2 of Ethylmalonyl-CoA decarboxylase

Decarboxylates ethylmalonyl-CoA, a potentially toxic metabolite, to form butyryl-CoA, suggesting it might be involved in metabolite proofreading (PubMed:22016388). Acts preferentially on (S)-ethylmalonyl-CoA but has also some activity on the (R)-isomer (By similarity). Also has methylmalonyl-CoA decarboxylase activity at lower level (By similarity).

Matrix Type

  • Tissue/Cells

Gene Symbol

  • ECHDC1

UniProt ID

  • Q9NTX5

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